Cell Biology Antibodies 9

Anti-CSTB Antibody (CAB3815)

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SKU:
CAB3815
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-CSTB Antibody
Antibody SKU:CAB3815
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC IF
Reactivity:Human, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-98 of human CSTB (NP_000091.1).
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Rat
Positive Samples:HepG2, SW480, DU145, A-549, A-431, MCF7
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-98 of human CSTB (NP_000091.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MMCG APSA TQPA TAET QHIA DQVR SQLE EKEN KKFP VFKA VSFK SQVV AGTN YFIK VHVG DEDF VHLR VFQS LPHE NKPL TLSN YQTN KAKH DELT YF
Gene ID:1476
Uniprot:P04080
Cellular Location:Cytoplasm, Nucleus
Calculated MW:11kDa
Observed MW:14kDa
Synonyms:CSTB, CPI-B, CST6, EPM1, EPM1A, PME, STFB, ULD
Background:The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). One type of mutation responsible for EPM1 is the expansion in the promoter region of this gene of a CCCCGCCCCGCG repeat from 2-3 copies to 30-78 copies.
UniProt Protein Function:CSTB: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1). EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Belongs to the cystatin family.
UniProt Protein Details:

Protein type:Inhibitor

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: extracellular space; cytoplasm; nucleolus

Molecular Function:protease binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity

Biological Process: negative regulation of proteolysis; regulation of apoptosis; adult locomotory behavior; negative regulation of peptidase activity

Disease: Myoclonic Epilepsy Of Unverricht And Lundborg

NCBI Summary:The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and kininogens. This gene encodes a stefin that functions as an intracellular thiol protease inhibitor. The protein is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. The protein is thought to play a role in protecting against the proteases leaking from lysosomes. Evidence indicates that mutations in this gene are responsible for the primary defects in patients with progressive myoclonic epilepsy (EPM1). [provided by RefSeq, Jul 2008]
UniProt Code:P04080
NCBI GenInfo Identifier:1706278
NCBI Gene ID:1476
NCBI Accession:P04080.2
UniProt Related Accession:P04080
Molecular Weight:11,140 Da
NCBI Full Name:Cystatin-B
NCBI Synonym Full Names:cystatin B (stefin B)
NCBI Official Symbol:CSTB  
NCBI Official Synonym Symbols:PME; ULD; CST6; EPM1; STFB; EPM1A  
NCBI Protein Information:cystatin-B; CPI-B; liver thiol proteinase inhibitor
UniProt Protein Name:Cystatin-B
UniProt Synonym Protein Names:CPI-B; Liver thiol proteinase inhibitor; Stefin-B
Protein Family:Cystatin
UniProt Gene Name:CSTB  
UniProt Entry Name:CYTB_HUMAN
Anti-CSTB Antibody (CAB3815)Western blot analysis of extracts of various cell lines, using CSTB antibody (CAB3815) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 10s.
Anti-CSTB Antibody (CAB3815)Immunohistochemistry of paraffin-embedded human prostate using CSTB antibody (CAB3815) at dilution of 1:100 (40x lens).
Anti-CSTB Antibody (CAB3815)Immunofluorescence analysis of HeLa cells using CSTB antibody (CAB3815) at dilution of 1:100. Blue: DAPI for nuclear staining.
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